尿IGFBP-7对危重症患儿急性肾损伤的早期预测价值

目的探讨尿胰岛素样生长因子结合蛋白7(IGFBP-7)对危重症患儿急性肾损伤(AKI)的早期预测价值。方法选择2012年5月至8月儿童重症监护病房(PICU)收治的患儿为研究对象。分为轻度AKI(AKI 1期)、严重AKI(AKI2和3期)和非AKI组。检测入PICU第一个24小时尿IGFBP-7水平,并于入PICU 24小时内行儿童死亡风险Ⅲ(PRISMⅢ)评分。以多因素logistic回归分析评估在校正混杂因素后尿IGFBP-7与AKI的关系,用受试者工作特征曲线(ROC)及曲线下面积(AUC)评价尿IGFBP-7对危重症患儿AKI的早期预测价值。结果共纳入危重症患儿144例,21例(14.6%)在样本采集120小时内发生AKI,其中严重AKI 11例。严重AKI组入PICU第一个24小时的尿IGFBP-7水平、PRISMⅢ评分均高于轻度AKI及无AKI组,差异均有统计学意义(P0.05)。Logistic回归分析显示,在校正年龄、体质量、PRISMⅢ评分后,尿IGFBP-7是严重AKI的独立危险因素(OR=2.93,95%CI:1.07～8.03,P=0.037),预测危重症患儿严重AKI的AUC值为0. 79(95%CI:0. 66～0. 92,P=0. 001)。结论尿IGFBP-7是危重症患儿严重AKI的独立预测指标,具有早期预测价值。     

1型糖尿病伴糖尿病酮症酸中毒患儿发生急性肾损伤的临床研究北大核心CSCD

目的评估1型糖尿病伴糖尿病酮症酸中毒(diabetic ketoacidosis,DKA)患儿发生急性肾损伤(acute kidney injury,AKI)的情况,探讨导致DKA患儿发生AKI的可能潜在因素。方法回顾性将2018年1月1日至2020年12月31日在南京医科大学附属儿童医院就诊的45例1型糖尿病伴DKA患儿,按照入院时是否合并AKI分为无AKI组(n=37)和合并AKI组(n=8)。收集两组患儿社会人口学资料,入院时的体检数据,包括身高、体重、血压和心率等,采用化学发光微粒免疫分析法测定患儿入院及出院时血肌酐和尿素氮等指标水平。采用多因素logistic回归模型分析1型糖尿病伴DKA患儿发生AKI的影响因素。结果45例患儿确诊中位年龄为9.2岁,8例(18%)入院时合并AKI的患儿中,6例为1期AKI,2例为3期AKI。血校正钠水平升高与1型糖尿病伴DKA患儿发生AKI密切相关(P<0.05),而入院时较高的胰岛素水平则不易发生AKI(P<0.05)。结论1型糖尿病伴DKA患儿AKI的发生率较高,临床上应积极纠正DKA,尽快控制血糖,并定期对这部分儿童病例进行肾功能复查和随访。[中国当代儿科杂志,2022,24(8):858-862]     

低白蛋白血症对心脏手术后患儿急性肾损伤发生的影响

目的 探讨小儿心脏病体外循环（CPB）手术后的低白蛋白血症对术后急性肾损伤（AKI）发生的影响。方法 回顾性分析2012~2016年行心脏病CPB手术患儿1 110例临床资料,按术后48 h内最低白蛋白浓度分为低白蛋白组（≤35 g/L）和正常白蛋白组（> 35 g/L）,比较两组患儿的围手术期资料和AKI发生率。对不均衡的围手术期各因素进行倾向评分匹配后再次比较AKI发生率。采用logistic回归分析术后AKI发生的围手术期危险因素。结果 术后AKI发生率为13.78%（153/1 110）,病死率为2.52%（28/1 110）,AKI患儿病死率为13.1%（20/153）,术后白蛋白≤35 g/L 的患儿占44.50%（494/1 110）。匹配前后低白蛋白组AKI发生率均比正常白蛋白组要高（P < 0.05）;匹配前后AKI患儿术后白蛋白浓度均低于非AKI患儿（P < 0.05）;Logistic多因素回归分析结果显示术后白蛋白≤35 g/L是术后AKI发生的独立危险因素之一。结论 术后48 h内白蛋白≤35 g/L是心脏CPB术后患儿AKI发生的独立危险因素,术后加强对白蛋白的检测和补充对控制术后AKI的发生有积极作用。     

流行性病毒感染致危重症儿童急性肾损伤临床分析

目的 分析流行性病毒感染危重症患儿急性肾损伤(AKI)的发生情况,并探讨血肌酐及尿量在AKI诊断中的意义.方法 回顾性分析急诊科住院的甲型H1N1流感病毒感染患儿(H1N1组)及肠道病毒EV71感染患儿(EV71组)临床资料.结果 共28例,H1N1组18例(男6例,女12例),平均年龄5.4岁,EV71组10例(男8例,女2例),平均年龄1.1岁.(1)H1N1组4例发生AKI,平均受累脏器5.3个,2例1期患儿治疗后痊愈;2例3期患儿死亡;14例未发生AKI,平均受累脏器3.0个,死亡4例.(2)EV71组:3例发生AKI(1期)者与3例血肌酐升高45.0％～ 47.6％者平均受累脏器5.7个,均死亡;4例无血肌酐升高者平均受累AKI脏器3.0个,病情好转.结论 流行性病毒感染所致危重症,发生AKI者受累脏器更多;H1N1感染AKI程度轻者早期积极干预预后相对好,程度重者预后差;E V71感染发生AKI则预后极差.诊断儿童AKI血肌酐较尿量的敏感性高.     

重型β地中海贫血儿童异基因造血干细胞移植后并发出血性膀胱炎的危险因素分析北大核心CSCD

目的探讨重型β地中海贫血(β-thalassemia major,TM)患儿异基因造血干细胞移植(allogeneic hematopoietic stem cell transplantation,allo-HSCT)后并发出血性膀胱炎(hemorrhagic cystitis,HC)的危险因素。方法回顾性分析2021年1月-2022年11月在深圳市儿童医院进行allo-HSCT的247例TM患儿的临床资料,以术后是否并发HC,分为HC组(91例)和非HC组(156例),采用多因素logistic回归分析探讨HC发生的危险因素,并采用受试者操作特征曲线分析相关因素预测HC的效能。结果247例allo-HSCT TM患儿中,HC发生率为36.8%(91/247)。单因素分析显示,年龄、供受者血型不一致、发生急性移植物抗宿主病(acute graft-versus-host disease,aGVHD)、尿BK病毒核酸(BK virus deoxyribonucleic acid,BKV-DNA)阳性和≥2种病毒感染与患儿allo-HSCT后并发HC有关(P<0.05)。多因素分析显示,供受者血型不一致(OR=3.171,95%CI:1.538~6.539)、发生aGVHD(OR=2.581,95%CI:1.125~5.918)和尿BKV-DNA阳性(OR=21.878,95%CI:9.633~49.687)是allo-HSCT TM患儿并发HC的独立危险因素。受试者操作特征曲线分析显示,单一尿BKV-DNA阳性或联合其他2种危险因素(发生aGVHD、供受者血型不一致)预测allo-HSCT后并发HC具有一定的准确性(曲线下面积>0.8,P<0.05)。结论供受者血型不一致、发生aGVHD和尿BKV-DNA阳性是TM患儿allo-HSCT后并发HC的独立危险因素,定期监测尿BKV-DNA对HC的早期诊断及治疗具有积极意义。     

重型地中海贫血患儿造血干细胞移植术后心包积液的临床特征及危险因素分析

目的探讨重型地中海贫血(TM)患儿异基因造血干细胞移植(allo-HSCT)术后心包积液发生的特点、危险因素及预后。方法病例对照研究。选择2012年1月至2020年12月在深圳市儿童医院血液肿瘤科造血干细胞移植中心行allo-HSCT的446例TM患儿为研究对象, 分析其心包积液发生情况, 根据是否发生心包积液分成心包积液组和非心包积液组, 分析TM患儿allo-HSCT术后心包积液发生的危险因素, 采用Kaplan-Meier法进行两组患儿的生存分析。结果 446例患儿中25例发生了心包积液, 发生率为5.6%, 心包积液发生的时间为移植后75.0(66.5, 112.5)d, 25例中有22例(88.0%)心包积液发生在移植半年以内, 有19例(76.0%)发生在移植100 d以内。所有患儿均通过心脏超声确诊心包积液, 其中仅1例发生心包填塞, 行急诊心包穿刺抽液治疗, 其他均为保守治疗。25例患儿经治疗心包积液均消失。TM患儿allo-HSCT术后心包积液的发生与性别、移植供者类型、输注的单个核细胞(MNC)数量、移植术后肺部感染及移植相关血栓性微血管病(TA-TMA)均有关(χ...     

儿童异基因造血干细胞移植术后巨细胞病毒感染临床分析

目的探讨儿童异基因造血干细胞移植(allo-HSCT)后巨细胞病毒(CMV)感染的危险因素及临床相关特征。方法收集2016年1月至2018年12月共269例allo-HSCT患儿的临床资料。监测移植后全血CMV-DNA拷贝数,分析移植患儿CMV感染发生率、发生时间、危险因素及预后。结果 269例患儿中,男167例、女102例,中位年龄65个月(33～115个月),其中165例发生CMV感染,感染率为61.3%,感染发生时间为移植后23 d(15～34 d),感染持续时间38 d(25～66 d)。Logistic回归分析发现患儿移植年龄65个月、移植后发生Ⅱ～Ⅳ级aGVHD是发生CMV感染的危险因素,而亲缘全相合移植能降低CMV感染发生风险(P0.05)。发生Ⅱ～Ⅳ级急性移植物抗宿主病(aGVHD)及使用脐血移植与发生难治性CMV感染相关(P0.05)。难治性CMV感染组与非难治性CMV感染组总体生存率及无病生存率的差异有统计学意义(P0.05)。结论移植患儿年龄大、Ⅱ～Ⅳ级aGVHD能增加CMV感染的发生风险,亲缘全相合移植能降低CMV感染的发生风险。脐血移植后易发生难治性CMV感染;难治性CMV感染初次检测到CMV感染时间早,峰值高。     

极低出生体质量儿急性肾损伤的危险因素及结局分析

目的探讨极低出生体质量(VLBW)儿发生急性肾损伤(AKI)的危险因素。方法回顾分析2012年1月至2016年12月收治的出生日龄≤3天的VLBW新生儿的临床资料。根据KDIGO改良的新生儿AKI标准,比较其中发生AKI(AKI组)与未发生AKI(NAKI组)新生儿的差异,并分析发生AKI的危险因素及AKI婴儿死亡的危险因素。结果 313例VLBW新生儿中126例发生AKI,发生率为40.3%。与NAKI组相比,AKI组的胎龄、出生体质量、5分钟Apgar评分、危重评分、平均动脉压更低,母亲年龄更大,发生胎膜早破更多,合并呼吸衰竭等发生率更高,入院时白细胞计数、降钙素原值更大,白蛋白、血钠更低,出生时有创机械通气更多,机械通气时间更长,病死率更高,差异均有统计学意义(P0.05)。多因素logistic回归分析发现,胎龄小、呼吸衰竭、出生时有创机械通气为VLBW新生儿发生AKI的独立危险因素;入院时酸中毒程度越重、伴随肺出血是AKI患儿死亡的独立危险因素。结论胎龄小、呼吸衰竭、出生时有创机械通气会显著增加VLBW新生儿发生AKI的风险,代谢性酸中毒程度重、伴随肺出血会显著增加AKI患儿的死亡风险。     

儿童造血干细胞移植后急性肾损伤相关危险因素的回顾性研究

目的 探讨造血干细胞移植（hematopoietic stem cell transplantation,HSCT）后急性肾损伤（acute kidney injury,AKI）的危险因素。 方法 回顾性研究2018年1月至2020年1月111例行HSCT患儿的临床资料。采用多因素logistic回归分析筛选出AKI发生的影响因素;采用Kaplan-Meier生存分析比较不同级别AKI患儿生存预后差异。 结果 111例HSCT患儿中,AKI发生率为52.3%（58/111）。移植物抗宿主病（Ⅱ~Ⅳ度）（OR=4.406,95%CI：1.501~12.933,P=0.007）、肝小静脉闭塞综合征（OR=4.190,95%CI：1.191~14.740,P=0.026）、血栓性微血管病（OR=10.441,95%CI：1.148~94.995,P=0.037）与HSCT患儿移植后AKI发生密切相关。AKI Ⅲ期患儿的1年生存率（28.6%±12.1%）低于NAKI（82.8%±5.2%）、AKI Ⅰ期（81.7%±7.4%）、AKI Ⅱ期（68.8%±11.6%）患儿（P<0.05）。 结论 患儿HSCT后发生Ⅲ期AKI具有较高的病死率;移植物抗宿主病、肝小静脉闭塞综合征、血栓性微血管病与HSCT后AKI发生密切相关。     

影响新生儿急性肾损伤预后的危险因素分析

目的分析新生儿急性肾损伤(AKI)的临床特点,了解影响新生儿AKI预后的危险因素。方法回顾分析2012年1月至2019年3月符合AKI诊断标准且临床资料齐全的105例新生儿的相关临床资料。按照治疗结局将患儿分为好转组和放弃或死亡组,比较两组临床特点,分析影响新生儿AKI预后的危险因素。结果共纳入105例AKI患儿,好转组48例、放弃或死亡组57例。与好转组相比,放弃或死亡组患儿的出生体质量低,极低出生体质量儿比例高,最低尿量值低,尿量1 mL/(kg·h)的比例高,休克、呼吸衰竭、凝血功能障碍、肺出血、消化道出血发生率高,使用呼吸机支持治疗的比例高,差异均有统计学意义(P0.05)。多元logistic回归分析结果显示,尿量1 mL/(kg·h)、合并呼吸衰竭是影响新生儿AKI预后的独立危险因素(OR=4.61,95%CI:1.68～12.63,P=0.003;OR=17.88,95%CI:5.61～56.98,P0.001)。结论新生儿AKI的病死率高,少尿、合并呼吸衰竭显著增加AKI新生儿发生不良预后的风险。     

Resurgence of measles in Singapore: profile of hospital cases

OBJECTIVE: To ascertain the profile of cases of measles seen at a general hospital during a recent outbreak that occurred despite a measles vaccination program. METHODOLOGY: A retrospective study from January 1991 to March 1998. All patients with measles (ICD code 055. 9) seen at the emergency unit or as inpatients were included. RESULTS: There were 87 cases identified. The diagnosis was clinical in all and proven serologically in 71%. Eighty-five per cent of the cases occurred between January 1997 and March 1998. There was a bi-modal age distribution with peaks in the very young (相似文献   

Allergic factors associated with the development of asthma and the influence of cetirizine in a double-blind, randomised, placebo-controlled trial: First results of ETA®

There is a common progression known as the allergic march from atopic dermatitis to allergic asthma. Cetirizine has several antiallergic properties that suggest a potential effect on the development of airway inflammation and asthma in infants with atopic dermatitis. Methods. Over a two year period, 817 infants aged one to two years who suffered from atopic dermatitis and with a history of atopic disease in a parent or sibling were included in the ETAC^® (Early Treatment of the Atopic Child) trial, a multi-country, double-blind, randomised, placebo-controlled trial. The infants were treated for 18 months with either cetirizine (0.25mg/ kg b.i.d.) or placebo. The number of infants who developed asthma was compared between the two groups. Clinical and biological assessments including analysis of total and specific IgE antibodies were performed. Results. In the placebo group, the relative risk (RR) for developing asthma was elevated in patients with a raised level of total IgE (≥ 30 kU/I) or specific IgE (≥ 0.35 kUA/I) for grass pollen, house dust mite or cat dander (RR between 1.4 and 1.7). Compared to placebo, cetirizine significantly reduced the incidence of asthma for patients sensitised to grass pollen (RR = 0.5) or to house dust mite (RR = 0.6). However, in the population that included all infants with normal and elevated total or specific IgE (intention-to-treat - ITT), there was no difference between the numbers of infants developing asthma while receiving cetirizine or placebo. The adverse events profile was similar in the two treatment groups. Discussion. Raised total IgE level and raised specific IgE levels to grass pollen, house dust mite or cat dander were predictive of subsequent asthma. Cetirizine halved the number of patients developing asthma in the subgroups sensitised to grass pollen or house dust mite (i.e. 20% of the study population). In view of the proven safety of the drug, we propose this treatment as a primary pharmacological intervention strategy to prevent the development of asthma in specifically sensitised infants with atopic dermatitis.     

A profile of respiratory symptoms in urban and rural South Australian school children

This report describes the cross-sectional analyses of data from the first year of a longitudinal study using questionnaire and respiratory function data over a 5 year period from a sample of rural South Australian school children. The cumulative or lifetime prevalences of respiratory symptoms were estimated in 825 rural and 1261 urban school children aged between 5 and 15 years in order to determine if the prevalence rates differed between rural and urban school children. The study found the overall cumulative prevalence of asthma and/or wheezy breathing (AWB) to be 24.1% in the rural school children compared to 27.6% in the urban school children. Most children developed AWB symptoms before the age of 7 years, with 20% reporting moderately severe symptoms and 10% having more than one attack per fortnight. The cumulative prevalence of bronchitis, loose/rattly cough (BLRC) differed significantly between the rural school children (34.1%) and urban school children (47.9%). The BLRC symptoms preceded the development of AWB in many cases. Urban school children also reported a higher prevalence of atopic conditions.     

Hauttemperaturen verschiedener Körperoberflächenareale des Rumpfes bei Säuglingen

Summary In two groups of infants (3–53 weeks old) skin temperatures were controlled in different areas of the trunk—i.e.: regions of sternum, lungs, heart, liver, spleen, kidneys—at different room-temperatures (group I: 21–25°C; group II: 29–32°C). Rectal temperatures of some probands in both groups also had been controlled simultaneously. A definite change in the reaction to heat was proofed in different periods of the first year of life. In higher environmental temperatures the skin temperature was almost constant at every controll-point of the skin, even in older infants. In lower environmental temperatures the skin temperatures lowered continuously with age till 7. to 9. moth. From 10. to 12. month the lowering of skin temperature discontinued. The rectal temperatures were relatively constant in all infants. Only in infants from 7. to 12. month, whose skin temperatures were controlled in lower as well as in higher environmental temperatures, a tendency to higher rectal temperatures was proofed in warmer environmental temperatures.The significance of these results is discussed.

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Untersuchungen mit Unterstützung durch die Deutsche Forschungsgemeinschaft.     

Psychopathologie du syndrome d’Asperger et « aspérigisation » de la société contemporaine

The author has attempted here to point out, just for a start, the characteristics of Asperger syndrome from the point of view of psychopathology through a rereading of Hans Asperger's original paper (1944). This thesis merits reevaluation, if for no other reason than to fill the gaps in operational diagnostics based on the DSM. It is found by rereading that Asperger's view of the principal disturbances of autistic psychopathy include a “disturbance of natural evidence” or a “crisis of common sense”. This question of natural evidence that he evokes with regard to autistic psychopathy corresponds to W. Blankenburg's natural evidence, which constitutes a key concept for comprehending schizophrenia in the form poor-symptom (“symptomarme Schizophrenie”) that he observes in the speech of his patient Anne Rau. One can deduce from this that in terms of fundamental disturbances, Asperger syndrome and this “symptom-poor” schizophrenia overlap at the level of loss of natural evidence. It is moreover possible to classify Asperger syndrome among the disturbances of spacing in the sense meant by the evolutionary psychiatry of A. Stevens and J. Price. The author then develops our comprehension of Asperger syndrome from the point of view of the perspective proposed by the notion of resilience in people with Asperger syndrome and of the possibility for them, through these mechanisms of adaptation, to find in the organization of the personality of the “as if” type a position of relative equilibrium. They concur or overlap in the creation of crutches, of borrowed personalities secondarily legitimated by the reaction of the socius. This will end up in the production of inventions and œuvres (works). Clearly, one rarely encounters several cases that one could consider pertinently to be “successful” Asperger syndrome. Finally, the author notes that one can find a sort of isomorphism between Asperger syndrome and contemporary society when he proposes the term “asperigisation” to characterize our society, given that the equilibrium between emotion and logic is strongly disturbed in these patients, in whom logic undergoes hypertrophy while emotion is impoverished. From this perspective, the author hopes to suggest reasons for the increase in the number of cases of Asperger syndrome in the clinical setting and in society in general in our contemporary era.     

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孤独症谱系障碍(autistic-spectrum disorders,ASDs)近年来患病率逐年攀升至1%左右,其症状往往伴随终生,成为严重威胁儿童健康和发展的神经发育性疾患;注意缺陷多动障碍(attention deficit hyperactivity disorder,ADHD)是儿童期最常见的精神障碍,国内报道患病率为4.13%～5.83%,其症状可延续至青少年期,甚至到成年期[1]。这两类精神障碍在成年期的临床表现、共患病、治疗策略和预后与儿童期有哪些不同呢?本文通过回顾相     

Infiltrations of Epstein-Barr virus-carrying cells in granular lymphocyte-proliferative disorders corresponding to chronic active Epstein-Barr virus infection

A 21-year-old man with granular lymphocyte-proliferative disorders (GLPD) associated with chronic active Epstein-Barr virus (EBV) infection is described. Chromosomal analyses revealed several clonal abnormalities and two of them were mainly repetitious. High copy numbers of monoclonal EBV genome were also detected in the proliferative large granular lymphocytes (LGLs), indicating the monoclonal expansion of EBV-infected LGLs. The patient had an indolent course for several years, and there was no evidence of infiltrations of his bone marrow until the end stage. At autopsy, microscopic studies revealed marked infiltrations of LGL in the liver and spleen, and the infiltrating cells were NK-cell immunophenotype. The infiltrated LGLs showed latency I.     

LUTEINIZING HORMONE RECEPTOR MUTATIONS IN DISORDERS OF SEXUAL DEVELOPMENT AND CANCER

Human male sexual development is regulated by chorionic gonadotropin (CG) and luteinizing hormone (LH). Aberrant sexual development caused by both activating and inactivating mutations of the human luteinizing hormone receptor (LHR) have been described. All known activating mutations of the LHR are missense mutations caused by single base substitution. The most common activating mutation is the replacement of Asp-578 by Gly due to the substitution of A by G at nucleotide position 1733. All activating mutations are present in exon 11 which encodes the transmembrane domain of the receptor. Constitutive activity of the LHR causes LH releasing hormone-independent precocious puberty in boys and the autosomal dominant disorder familial male-limited precocious puberty (FMPP). Both germline and somatic activating mutations of the LHR have been found in patients with testicular tumors. Activating mutations have no effect on females. The molecular genetics of the inactivating mutations of the LHR are more variable and include single base substitution, partial gene deletion, and insertion. These mutations are not localized and are present in both the extracellular and transmembrane domain of the receptor. Inactivation of the LHR gives rise to the autosomal recessive disorder Leydig cell hypoplasia (LCH) and male hypogonadism or male pseudohermaphroditism. Severity of the clinical phenotype in LCH patients correlates with the amount of residual activity of the mutated receptor. Females are less affected by inactivating mutation of the LHR. Symptoms caused by homozygous inactivating mutation of the LHR include polycystic ovaries and primary amenorrhea.     

EXCITING NEW TREATMENT APPROACHES FOR PATHYPHYSIOLOGIC MECHANISMS OF SICKLE CELL DISEASE

During the past several decades, our understanding of the complex pathophysiology of vasoocclusion associated with sickle cell disease has improved greatly. Interaction of genes, hemoglobin molecules, red cell membrane and metabolic changes, cell-cell interactions and cell-plasma interactions, red cell adhesion to vascular endothelium, activation of coagulation, and vascular reactivity play a role in vaso occlusion. Penicillin prophylaxis of pneumococcal infections and appropriate use of blood transfusions and other supportive measures improved survival of sickle cell patients. Hydroxyurea made a major impact on sickle cell therapy when it was shown to decrease acute painful episodes, acute chest syndrome, and the need for blood transfusion in adults. Significant experience in the use of hydroxyurea has been accumulated in older children. The benefits and risks of hydroxyurea for younger children and long-term risks in all patients will be evaluated in future investigations. Other promising therapies include butyrate compounds, clotrimazole, magnesium supplementation, poloxamer 188, antiadhesion agents, anticoagulant approaches, and nitric oxide. Hemopoietic transplantation remains the only curative therapy. However, several transgenic mouse models are available for studies of gene therapy or other treatment approaches on biochemical, cellular, and pathologic effects of mutant genes.